BCNS (Basal Cell Nevus Syndrome) also called Gorlin syndrome or nevoid basal cell carcinoma, is a condition that affects many areas of the body and increases the risk of developing various cancerous and noncancerous tumors. In people with Gorlin syndrome, the type of cancer diagnosed most often is basal cell carcinoma, which is the most common form of skin cancer. Individuals with Gorlin syndrome typically begin to develop basal cell carcinomas during adolescence or early adulthood. These cancers occur most often on the face, chest, and back.
The number of basal cell carcinomas that develop during a person’s lifetime varies among affected individuals. Some people with Gorlin syndrome never develop any basal cell carcinomas, while others may develop thousands of these cancers. Individuals with lighter skin are more likely to develop basal cell carcinomas than are people with darker skin.
Mutations in the PTCH1 gene are the main cause of Gorlin syndrome. The PTCH1 gene is a tumor suppressor gene, which means it stops cells from proliferating too rapidly or in an uncontrolled way. Mutations in this gene prevent the production of patched-1 or lead to the production of an abnormal version of the receptor. An altered or missing patched-1 receptor cannot effectively suppress cell growth and division. As a result, cells proliferate uncontrollably to form the tumors that are characteristic of Gorlin syndrome.
As per the study conducted by American Osteopathic College of Dermatology, the most identifiable feature of NBCCS is multiple basal cell carcinomas, a common type of skin cancer. This type of skin cancer is most often seen in older adults after many years of sun damage. People with NBCCS frequently get basal cell carcinomas in their 20s and 30s, though can be as young as 3 to 4 years old. They also have many more basal cell cancers.
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